Facts about Stiff Person Syndrome

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Fact #13

There are 5 types of Stiff Person Syndrome:

-classic stiff person syndrome
-focal stiff person syndrome
-jerking stiff person syndrome—scribblesdizzle has this type (see her post above)
-progressive encephalomyelitis with rigidity and myoclonus (PERM)
-paraneoplastic-related stiff person syndrome

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Fact #14

According to raresiseases.org

“
There are classes of medications that should be avoided in SPS, including serotonin-norepinephrine reuptake inhibitors (SNRIs; i.e, tricyclic antidepressants and duloxetine) and opioids. SNRIs have previously been shown to worsen the EMG activity and clinical symptoms in SPS. Opioids are not recommended for pain control because most individuals with SPS are on benzodiazepines. Mixing these two classes of medications can lead to severe respiratory depression and death.

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Fact #15

It’s thought that Stiff Person Syndrome is caused by the immune system producing antibodies to a protein called GAD. GAD is necessary to synthesize GABA. GABA is necessary to control muscle movement and to inhibit excitability in the brain and spinal cord.

This means people with SPS will have rigid muscles and spasms. They will also find themselves working harder to inhibit emotional outbursts. They will startle in an obvious way, often experiencing stiffness or severe spasms in response to a stimulation that may almost go unnoticed, or at least rapidly accommodated to by others.

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Fact #16

There is a case study showing that medical marijuana (Sativex) can alleviate some Stiff Person Syndrome symptoms:

http://ejhp.bmj.com/content/19/2/219.2

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Fact #17

Most, but not all, of patients with Stiff Person Syndrome have elevated GAD65 levels in the blood indicates that the body is creating an antibody that attacks GAD, impairing the body’s ability to make GABA.

However, Some people with stiff person syndrome have antibodies to amphiphysin, which is a protein involved in the transmission of signals from one neuron to another. People with these antibodies have a higher risk for developing breast, lung, or colon cancer.

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Fact #18

Both Type 1 Diabetes and Stiff Person Syndrome are autoimmune disorders with elevated GAD in serum. But the GAD in SPS is 10-1,000 fold higher than in Type 1 Diabetes,.

A large number of people with SPS, about 35%, do have, or go on to develop Type 1 Diabetes.

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Fact #19

There are complementary therapies that can benefit Stiff Person Therapy:
Passive PT , myofascia release, soaking and moving around in warm water, not cold water.
Ultrasound
Acupuncture

Listen to Dr. Newsome talk about this in the Management of Stiff Person Syndrome window of this web page:

https://www.hopkinsmedicine.org/neur...ome/index.html

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Fact #20

There is no reported research on a standard physical therapy approach for Stiff Person Syndrome, only case reports. PT thus have to create their own approach within general guidelines.

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Fact #21

Any genetic factors involved in causing stiff person syndrome have not been found . there have been cases of multiple people in the same family being affected by SPS, however.

It may be that a genetic mutation combined with environmental event results in Stiff Person Syndrome.

— this summarizes statement on rare diseases at NIH

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Fact #22


5% of all SPS patients have the Paraneoplastic variant of Stiff Person Syndrome. For them, there are anti-amphiphysin and anti-gephyrin antibodies ),most commonly found in association with breast adenocarcinoma and small-cell lung carcinoma.